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|Motor neuron disease|
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The motor neuron diseases (MND) are a group of neurological disorders that selectively affect motor neurons, the cells that control voluntary muscle activity including speaking, walking, breathing, swallowing and general movement of the body. They are generally progressive in nature, and cause progressive disability and death.
Terminology[edit | edit source]
Terms used to describe the motor neuron diseases can be confusing; in the UK "motor neuron disease" (with "neuron" sometimes spelt "neurone") refers to both amyotrophic lateral sclerosis (the most common form of disease) and to the broader spectrum of motor neuron diseases including progressive muscular atrophy, primary lateral sclerosis, and progressive bulbar palsy. In the United States the most common terms used are ALS (both specifically for ALS and as a blanket term) or "Lou Gehrig's disease". To avoid confusion, the annual scientific research conference dedicated to the study of MND is called the International ALS/MND Symposium. Although MND refers to a specific subset of pathologically similar diseases; there are numerous other afflictions of motor neurons that are pathologically distinct from MND and have a different clinical course. Examples of other diseases of the motor neuron that should not be confused with MND include spinobulbar muscular atrophy, spinal muscular atrophy, Charcot-Marie-Tooth disease, and many others.
Classification[edit | edit source]
There are five recognized subtypes of motor neuron diseases. They are distinguished by the nerve cells affected (upper and lower motor neuron), and the symptoms that result from this damage:
|Type||UMN degeneration||LMN degeneration|
|Amyotrophic lateral sclerosis (ALS)||yes||yes|
|Primary lateral sclerosis (PLS)||yes||no|
|Progressive muscular atrophy (PMA)||no||yes|
|Progressive bulbar palsy (PBP)||no||yes – bulbar region|
|Pseudobulbar palsy||yes – bulbar region||no|
The term "bulbar region" in the above table refers to the mouth, face, and throat.
The disease spinal muscular atrophy (SMA) as well as various other spinal muscular atrophies are classified as motor neuron diseases by the disease terminology classification system Medical Subject Headings (MeSH) but not by the tenth International Statistical Classification of Diseases and Related Health Problems (ICD-10) published in 1992; hence, they are not discussed in this article.
See also[edit | edit source]
- Amyotrophic lateral sclerosis
- Primary lateral sclerosis
- Progressive muscular atrophy
- Motor Neurone Disease Association
- Category:People with motor neurone disease
- Category:Deaths from motor neurone disease
References[edit | edit source]
- Motor Neuron Diseases Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS). www.ninds.nih.gov. URL accessed on 2010-11-07.
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